Beatriz Beitler

Secondary Chronic Myelogenous Leukemia: A Diverse Pathogenesis?

A patient with myasthenia gravis and a thymoma did not respond to thymectomy. He was submitted to radiotherapy concurrent with steroid therapy followed by an alkylating based chemotherapy. Four years later, he developed an otherwise typical Philadelphia chromosome/BCR-ABL positive chronic myelogenous leukemia (CML) that quickly evolved to a blast crisis. We discuss the possible cause-effect mechanism between the previous treatment and CML, and suggest that a distinct mechanism, albeit unknown, could be involved in the development and progression of secondary CML.

Aplicação do índice prognóstico internacional em pacientes com linfoma difuso de grandes células B em uma instituição brasileira

Diffuse Large B-Cell Lymphomas (DLBCL) correspond to 50% of non-Hodgkins lymphomas. Since 1993 the treatment of these patients has been directed by the International Prognostic Index (IPI), validated in several studies. However, the use of the IPI has not been evaluated in our population and social-economical conditions. In this study, we evaluate the impact of the age-adapted IPI (aIPI) in the complete response, overall survival and disease-free survival in under 60-year-old DLBCL sufferers treated in the Hematology Service of HCFMUSP. Of the 111 evaluated patients, 60 were classified as aIPI low and intermediate risk and 51 as aIPI intermediate-high and high risk. The patients with low and intermediate risk were analyzed as a whole with adapted low risk and patients with intermediate-high and high risk with the adapted high risk. We verified that the overall survival and disease-free survival were influenced by the clinic stage, LDH value and patients performance status. We recommend the regular use of IPI in the treatment of the patients with DLBCL in our institution. Rev. bras. hematol. hemoter. 2005;27(1):27-30.Diffuse Large B-Cell Lymphomas (DLBCL) correspond to 50% of non-Hodgkins lymphomas. Since 1993 the treatment of these patients has been directed by the International Prognostic Index (IPI), validated in several studies. However, the use of the IPI has not been evaluated in our population and social-economical conditions. In this study, we evaluate the impact of the age-adapted IPI (aIPI) in the complete response, overall survival and disease-free survival in under 60-year-old DLBCL sufferers treated in the Hematology Service of HCFMUSP. Of the 111 evaluated patients, 60 were classified as aIPI low and intermediate risk and 51 as aIPI intermediate-high and high risk. The patients with low and intermediate risk were analyzed as a whole with adapted low risk and patients with intermediate-high and high risk with the adapted high risk. We verified that the overall survival and disease-free survival were influenced by the clinic stage, LDH value and patients performance status. We recommend the regular use of IPI in the treatment of the patients with DLBCL in our institution.

Linfoma primário do sistema nervoso central

O linfoma primario do sistema nervoso central (LPSNC) e um linfoma extralinfonodal que, ao diagnostico, encontra-se restrito ao parenquima cerebral, as meninges e/ou cordao espinhal e/ou olhos. Sua incidencia triplicou nas ultimas tres decadas para 0,4 casos por 100.000 habitantes, representando 4% dos tumores do sistema nervoso central (SNC). Embora pacientes infectados pelo HIV tenham 3.600 vezes maior risco para o desenvolvimento do LPSNC, a incidencia nao aumentou apenas neste grupo de pessoas. Dados sugerem reducoes da incidencia de LPSNC em pacientes infectados apos a introducao de drogas anti-retrovirais. Cerca de 90% dos casos de LPSNC sao classificados como linfoma difuso de grandes celulas B, 10% tem envolvimento ocular e 10% sao HIV positivos. A apresentacao clinica depende da localizacao tumoral, prevalecendo os sintomas neurologicos em detrimento aos sistemicos. Os exames de tomografia computadorizada (TC) e ressonância nuclear magnetica (RNM) sao essenciais para o diagnostico, porem o exame confirmatorio deve ser o anatomopatologico. O estadiamento deve ser feito com exames de imagem e biopsia de medula ossea (BMO) bilateral. Os principais fatores de mau prognostico sao: performance status do paciente acima de 1, idade superior a 60 anos, DHL elevada, hiperproteinorraquia e acometimento de area cerebral nao hemisferica. Alguns fatores de prognostico biologicos tambem podem influenciar na sobrevida, a exemplo da expressao de Bcl-6, que confere melhor prognostico. O tratamento de escolha e a combinacao de quimioterapia contendo altas doses de metotrexate e radioterapia (RDT). Devido as altas taxas de neurotoxicidade associada a RDT, seu uso tem ficado mais restrito aos pacientes idosos, e os recidivados ou refratarios.

A case of TCR γδ + T-ALL with cross-lineage CD19 expression

A short while ago a close family friend, aged 24 years, died a month after a technically successful allogeneic bone marrow transplant for acute lymphocytic leukaemia in a London teaching hospital. The cause of death was fulminant adult respiratory distress syndrome apparently precipitated by toxoplasmosis. During the last 2 weeks of his life whilst on steroids he developed a low-grade fever with increasing C-Reactive Protein levels and was treated by the standard modalities of changing his Hickman line and using an escalating course of antibiotics and antifungals as per the Haematology Unit’s protocol. Toxoplasmosis was not considered in the differential diagnosis even though he was known to be seropositive and the donor seronegative. After reviewing the literature it has become clear to me that there is some uncertainty about the treatment of toxoplasmosis in the bone marrow transplant setting. I would like to ask my clinical colleagues who specialise in the field of bone marrow transplants if they could help me with the following questions: Is there a different clinical approach between the British and French to patients undergoing bone marrow transplants for leukaemia who are seropositive for toxoplasmosis? Why are patients in Britain who are seropositive for toxoplasmosis and who receive a bone marrow transplant not routinely given prophylaxis, as appears to be the practice in France? Does seropositivity pose an increased risk to a patient who undergoes chemotherapy followed by conditioning with total body irradiation and anti-T cell monoclonal antibodies and who is then transplanted with cells from a seronegative donor?2 What percentage of bone marrow transplant patients currently die of toxoplasmosis? Of those who die of ARDS or organ failure or of lung infections how many actually have autopsies which are then specifically examined for toxoplasmosis?3 What is the current prevalence of seropositivity for toxoplasmosis in Britain today? The current data seems to